AB0574 LEVEL OF AGREEMENT ON CORE SIGNS OF CUTANEOUS LUPUS ERYTHEMATOSUS (CLE) SUBTYPES AMONG DERMATOLOGISTS AND RHEUMATOLOGISTS AND DEVELOPMENT OF A CONCEPTUAL MODEL FOR CLE
Authors: P. Kamudoni, A. Kuhn, J. F. Merola, J. Wenzel, V. Werth, A. Bellenger, A. De Palma, P. Koochaki, J. Stansbury, C. Vazquez Mateo, S. Roy, E. Thomas, A. Lauer, O. Guenther, Y. Sun, A. G. Rodriguez
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> CLE can present as an isolated skin disease or with systemic lupus erythematosus (SLE) and is classified as chronic (CCLE; primarily discoid [DLE]), subacute (SCLE), intermittent (ICLE), or acute (ACLE). Except for erythema, skin-related signs/symptoms may vary by subtype. We conducted mixed methods research to refine and develop an optimal clinician-reported outcome measure for CLE trials. <h3>Objectives</h3> Evaluate the level of agreement among dermatologists and rheumatologists on CLE signs/symptoms and develop a conceptual model for CLE. <h3>Methods</h3> We conducted one-to-one interviews with practicing, board-certified dermatologists or rheumatologists followed by a rank-and-sort exercise, and a literature review. Interviews included open-ended and semi-structured questions on CLE diagnosis and their patients' signs/symptoms, and data were evaluated by thematic analysis. Two quantitative activities were conducted: first, clinicians sorted a list of skin signs/symptoms as 1) not characteristic, 2) moderately characteristic, or 3) highly characteristic of CLE or cutaneous SLE disease activity. Second, they classified CLE signs/symptoms as most closely associated with CCLE/DLE, SCLE or ACLE. Data were analyzed using an informal (ranking task) or formal (classification task) consensus model. <h3>Results</h3> Eleven dermatologists and 10 rheumatologists (US n=13; Europe n=8) were interviewed. Most dermatologists (70%) had 5–10 years' experience managing/treating patients with CLE; 56% of rheumatologists had 21–25 years' experience. The most frequently mentioned signs included erythema/rash (100%), malar rash (71%), palpable lesions (57%) and scale (57%). Photosensitivity (76%), itch (62%) and skin pain (57%) were the most frequently mentioned symptoms. Of the 33 skin signs presented, both specialties ranked photosensitive rash (weighted average: dermatologists 3.00; rheumatologists 2.85) and erythema (2.92 and 2.81) highest. However, some key differences were identified. For example, dermatologists ranked polycyclic (2.72) and annular (2.61) lesions (typical of SCLE) as highly characteristic of CLE, but rheumatologists ranked them as low or moderately characteristic (1.55 and 2.23, respectively). Also, dermatologists ranked markers of DLE, such as follicular plugging (2.88 and 2.36), higher than rheumatologists. Consensus analysis showed robust agreement between dermatologists (eigenvalue ratio 17.95; average competence 0.86) and modest agreement between rheumatologists (6.13; 0.67) in classifying skin manifestations by CLE subtype. Manifestations with the strongest agreement included acute alopecia (ACLE n=20; SCLE n=0; CCLE/DLE n=1), malar rash (n=21; n=0; n=0, respectively), dyspigmentation without scarring (n=1; n=18; n=2), psoriasiform annular plaques (n=0; n=20; n=1), dyspigmentation with scarring (n=0; n=0; n=21), and irreversible scarring alopecia (n=0; n=0; n=21). Variation was evident for signs more often associated with SCLE, including non-scarring photosensitive dermatitis (n=8; n=13; n=0). Multidimensional scaling showed notable differences in consensus between dermatologists and rheumatologists; while coherent clusters of symptoms were mapped to the CLE subtypes by dermatologists, the pattern for rheumatologists was more diffuse, suggesting a less consistent conceptualization of SCLE and CCLE. These results, together with the literature review, were used to develop a conceptual model for evaluating CLE. <h3>Conclusion</h3> Despite agreement on the importance of erythema and characterization of CLE as a photosensitive rash, differences between the specialties were apparent when rating CLE signs that typically accompany non-systemic, cutaneous disease. This might reflect increased training among dermatologists in clinical-histopathologic description of lesions. The conceptual model proposed will facilitate efforts to design measurement approaches to evaluate disease activity in clinical research and practice. <h3>Acknowledgements</h3> This study was sponsored by Merck Healthcare KGaA, Darmstadt, Germany, who funded medical writing support by Bioscript Group Ltd, Macclesfield, UK. ICON Clinical Research LLC were contracted to conduct the study but no payments were made for authorship. <h3>Disclosure of Interests</h3> Paul Kamudoni Employee of: Merck Healthcare KGaA, Darmstadt, Germany, Annegret Kuhn: None declared, Joseph F. Merola: None declared, Jörg Wenzel: None declared, Victoria Werth: None declared, Alex Bellenger Consultant of: Merck Healthcare KGaA, Darmstadt, Germany, Andrea De Palma Consultant of: Merck Healthcare KGaA, Darmstadt, Germany, Patricia Koochaki Consultant of: Merck Healthcare KGaA, Darmstadt, Germany, James Stansbury Consultant of: Merck Healthcare KGaA, Darmstadt, Germany, Cristina Vazquez Mateo Employee of: EMD Serono Research &amp; Development Institute, Inc., Billerica, MA, USA, an affiliate of Merck KGaA, Sanjeev Roy Employee of: Ares Trading SA, Eysins, Switzerland, an affiliate of Merck KGaA, Erik Thomas Employee of: EMD Serono Research &amp; Development Institute, Inc., Billerica, MA, USA, an affiliate of Merck KGaA, Alexandra Lauer Employee of: Merck Healthcare KGaA, Darmstadt, Germany, Oliver Guenther Employee of: Merck Healthcare KGaA, Darmstadt, Germany, Ying Sun Employee of: Merck Healthcare KGaA, Darmstadt, Germany, Almary Guerra Rodriguez Consultant of: Merck Healthcare KGaA, Darmstadt, Germany.